|Other Names||2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDE1B, BCKDH E1-beta, BCKDHB|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).|
|Cellular Location||Mitochondrion matrix.|
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Provided below are standard protocols that you may find useful for product applications.
Branched-chain keto acid dehydrogenase is a multienzymecomplex associated with the inner membrane of mitochondria, andfunctions in the catabolism of branched-chain amino acids. Thecomplex consists of multiple copies of 3 components: branched-chainalpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2)and lipoamide dehydrogenase (E3). This gene encodes the E1 betasubunit, and mutations therein have been associated with maplesyrup urine disease (MSUD), type 1B, a disease characterized by amaple syrup odor to the urine in addition to mental and physicalretardation, and feeding problems.
Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010) :Gorzelany, K., et al. Turk. J. Pediatr. 51(2):97-102(2009)Quental, S., et al. Mol. Genet. Metab. 94(2):148-156(2008)Kang, H., et al. Fertil. Steril. 89(3):728-731(2008)Flaschker, N., et al. J. Inherit. Metab. Dis. 30(6):903-909(2007)
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